Mastocytosis is a rare neoplasm characterized by the expansion and accumulation of mast cells in various organ systems. Systemic mastocytosis (SM) may or may not present with cutaneous lesions. To examine the frequency and clinical impact of cutaneous involvement, data on 1,510 patients with mastocytosis collected in the registry of the European Competence Network on Mastocytosis were analyzed. Cutaneous involvement was found in 1,195 of 1,510 patients (79.1%). Of these, 286 had cutaneous mastocytosis, and 721 had SM with skin involvement. Adult patients with skin involvement who did not have a bone marrow examination (n = 188) were defined as having mastocytosis in the skin. In 315 patients, SM without skin involvement was found. The percentage of cases with cutaneous involvement was higher in indolent SM (100%) and smoldering SM (87.9%) compared to aggressive SM (46.8%) or mast cell leukemia (38.5%). After a median follow-up of 5.6 years, no patient with cutaneous mastocytosis had died, but 2.6% of the patients with mastocytosis in the skin, 5.7% of the patients with SM with skin involvement, and 28.95% of the patients with SM without skin involvement had died. Overall survival was longer in patients with skin involvement (cutaneous mastocytosis and/or mastocytosis in the skin and/or SM with skin involvement) than in patients with SM without skin involvement (P < 0.0001). These data argue for a thorough examination of both the skin and bone marrow in adult patients with mastocytosis.

Objective To determine the type and frequency of physical therapy (PT) prescribed by physicians for patients in the registry of the German Network for Systemic Sclerosis. Methods The data for 4,252 patients were analyzed using descriptive statistics, chi-square tests, and odds ratios (ORs). Results Overall, 37.4% of patients (1,590 of 4,252) receivedPTat the end of a yearly follow-up. The most frequently used type ofPTwas lymphatic drainage (n = 1,061, 36.8%), followed by exercise therapy (n = 1,047, 36.3%) and heat therapy (n = 689, 23.9%). More than three-fourths of treated patients (82%) received 1 or 2 different forms ofPTsimultaneously. The prescription ofPTwas associated with the extent of skin fibrosis as measured by the modified Rodnan skin thickness score (<10 [41.8% of patients], 11-20 [55.8% of patients], and >21 [63.9% of patients];P< 0.001). Patients with musculoskeletal involvement (e.g., arthritis, muscle weakness, joint contractures, tendon friction rubs) had a higher chance of receivingPTthan patients without these symptoms, with correspondingORs ranging from 1.96 (95% confidence interval [95%CI] 1.69-2.28) for joint contractures to 3.83 (95%CI2.89-5.08) for arthritis. When comparing the type ofPTprescription across the initial and all follow-up visits from 2003 to 2017, significant alterations with a decreasing frequency of patients receivingPTcould be observed (P= 0.001). Conclusion To our knowledge, this is the first study reporting the use ofPTin patients with systemic sclerosis (SSc) in a large cohort. AlthoughSSc is characterized by considerable disability and restriction of motion, PT.

1325 © 2020 The Authors. Journal der Deutschen Dermatologischen Gesellschaft published by John Wiley & Sons Ltd on behalf of Deutsche Dermatologische Gesellschaft. | JDDG | 1610-0379/2020/1811 Die charakteristische primäre Läsion der Lyme-Borreliose (LB) ist das Erythema migrans (EM), das meist klinisch diagnostiziert werden kann [1]. Es wurden jedoch unterschiedliche Muster mit verschiedenen klinischen, mikrobiologischen sowie serologischen Befunden beschrieben [2]. Das vorherrschende histologische Bild ist ein oberflächliches und tiefes perivaskuläres und interstitielles Infiltrat, das sich hauptsächlich aus Lymphozyten, Plasmazellen und Eosinophilen zusammensetzt [3].

The characteristic primary lesion in Lyme borreliosis (LB) is erythema migrans (EM) which can be diagnosed clinically in the majority of cases [1]. However, different patterns have been described with various clinical, microbiological or serologic findings [2]. The predominant histologic picture is a superficial and deep perivascular and interstitial infiltrate composed mostly of lymphocytes, plasma cells and eosinophils [3].

OBJECTIVE:Scleroderma renal crisis (SRC) is a severe life-threatening manifestation in patients with systemic sclerosis (SSc). However, the knowledge about risk factors for SRC is limited. We determined here the frequency of SRC and identified risk factors for the prediction of SRC.METHODS:Based on regular followup data from the German Network for Systemic Scleroderma, we used univariate and multivariate generalized estimating equations to analyze the association between clinical variables, SSc subsets, therapy [i.e., angiotensin-converting enzyme inhibitors (ACEi), corticosteroids], and the occurrence of SRC.RESULTS:Data of 2873 patients with 10,425 visits were available for analysis with a mean number of registry visits of 3.6 ± 2.8 and a mean time of followup of 3.6 ± 3.8 years. In total, 70 patients developed SRC (70/2873, 2.4%). Of these patients, 57.1% (40/70) were diagnosed with diffuse cutaneous SSc, 31.4% (22/70) with limited cutaneous SSc, and 11.4% (8/70) with SSc-overlap syndromes. Predictive independent factors with the highest probability for SRC were positive anti-RNA polymerase antibodies (RNAP), a history of proteinuria prior to SRC onset, diminished DLCO, and a history of hypertension. Interestingly, positive antitopoisomerase autoantibodies did not predict a higher risk for SRC. Further, patients with SRC were significantly more frequently treated with ACEi and corticosteroids without being independently associated with SRC.CONCLUSION:In this cohort, SRC has become a rare complication. By far the highest risk for SRC was associated with the detection of anti-RNAP and proteinuria.

in assessing NB-UVB-induced response in vitiligo, and may also be used as a marker in monitoring disease progression.

Hautbezogene Scham ist ein wichtiger Aspekt der psychischen Belastung dermatologischer Patienten. Aufgrund des Mangels an robusten Erhebungsmethoden wird diese Art der Scham in der Forschung und der klinischen Praxis jedoch kaum beachtet. Ziel der Studie war es, hautbezogene Scham erstmals mittels einer deutschen Version der Skin Shame Scale (SSS-24) zu messen und diese anhand etablierter psychometrischer Parameter zu validieren. Der SSS-24-Fragebogen wurde 488 dermatologisch gesunden (DG) Personen (66,6 % Frauen) und 339 dermatologischen Patienten (DP) (66,7 % Frauen) vorgegeben. Zudem wurden Komponenten allgemeiner Scham (SHAME) und die psychische Symptombelastung (BSI[Brief Symptom Inventory]-18) erfasst. Die SSS-24 zeigte in beiden Stichproben adäquate psychometrische Eigenschaften und eine hohe innere Konsistenz (DG: α = 0,91; DP: α = 0,95). DP berichteten über mehr hautbezogene Scham als die DG (p < 0,001), aber in beiden Gruppen ging ein höheres Ausmaß an hautbezogener Scham mit höherer allgemeiner Scham sowie einer höheren psychischen Symptombelastung (p < 0,001) einher. Insgesamt konnten die Kennwerte der englischen Originalversion sehr gut in der deutschen Version der SSS-24 repliziert werden. Aufgrund der Zusammenhänge mit allgemeiner Scham und psychischer Symptombelastung sollte hautbezogene Scham zukünftig im psychodermatologischen Kontext verstärkt Berücksichtigung finden. Die SSS-24 stellt ein wertvolles Instrument zur Erforschung von Scham bei dermatologischen Erkrankungen und zur Erfassung des Bedarfs an psychosozialen Interventionen bei dermatologischen Patienten dar.

Background Attachment and spirituality are thought to have deep evolutionary roots but are always interpreted within the framework of culture, religion and personal beliefs. While insecure attachment has been observed to be positively related with psychopathology, a positive mental health effect has often been described for spirituality. To examine the cross-cultural validation of previous research focused on Austrian young adults with Western socialization, we attempt to replicate our study examining the influence spirituality has on the connection between insecure attachment and mood-related psychiatric burden with Indian young adults. Methods We investigated Avoidant (AV) and Anxious (AX) Attachment (ECR-RD), Religious (RWB) and Existential (EWB) Well-Being (MI-RSWB), and mood-related psychiatric burden (Anxiety, Depression, Somatization; BSI-18) in 443 (31% female) Indian young adults (age range: 18–30 years) with a Hindu upbringing. Results Compared to young adults with a Roman Catholic upbringing in a Western socialization, Indian participants did not differ in AX and EWB but scored higher in mood-related psychiatric burden ( eta 2 = .04), AV ( eta 2 = .14), as well as RWB ( eta 2 = .28; all p < .01). As in previous research only AX ( β = .40) positively predicted mood-related psychiatric burden ( ΔR 2 = .15 , all p < .01), while EWB was an additional negative predictor ( β = −.11, p < .05). Conclusions Our findings emphasize the universal importance of attachment and spirituality for mental health as well as the potential influence of socialization on their development. Furthermore, they underline that Existential Well-Being – including hope for a better future, forgiveness, and the experience of sense and meaning – appears to have a compensating effect on the relation between insecure attachment and impaired mental health.

Background: Data from prospective clinical trials support the use of immunomodulatory therapies (IT) for treatment of SSc-ILD. However, outcomes for SSc-ILD in respect to IT use in large real-life cohorts has only sparsely been reported. Methods: The German Network for Systemic Scleroderma (DNSS), which includes SSc pts. prospectively, was analyzed for SSc-ILD. Patients were categorized in IT vs. no-IT users and outcome was assessed. Results: SSc-ILD was reported in 1886 out of 4306 pts. 1109 used IT while 777 did not. Baseline characteristics at ILD diagnosis were similar with regards to gender, FVC (no IT 82% vs. IT 78%, p=0.117) and use of PH drugs. Significant differences in no-IT vs. IT were found for age, time since SSc diagnosis (10 vs. 7 years p<0.001), SSc subtype (p<0.001, diffuse 45% vs. 53%), DLCO (62% vs. 58%, p<0.001), mRSS (10.8 vs. 12.3, p=0.005), specific organ involvements (e.g. esophagus p=0.002, kidneys p=0.002), auto-antibody profile, and steroid use (33% vs. 56%, p<0.001). Disease progression (defined as either death or decline of FVC≥10% or DLCO≥15%) did not differ between groups (27.8% vs. 28.6%, p=0.712). All-cause mortality was similar with 14.6% for no-IT and 13.9% for IT. Also decline of FVC≥10% with 34% in the no-IT and 28% in the IT group (p=0.24) and of DLCO≥15% (35.5% vs. 30%, p=0.088) were comparable. A multinomial logistic regression model revealed an increased risk for the development of SSc-ILD by factor 1.16 if IT was applied (p=0.065). Conclusions: In this large real-life cohort of SSc-patients, the use of immunomodulatory therapies had no significant impact on outcomes in SSc-ILD. Yet, differences in baseline characteristics have to be taken into account.

Shame has been registered as a part of psychosocial distress in patients with psoriasis. This study investigated 44 patients with psoriasis and 88 age- and sex-matched individuals without skin disease. Skin shame, multifarious expressions of shame, psychological symptoms, overall health, dermatological quality of life, disease burden and disease severity were measured. Higher levels of skin shame correlated with a greater disease burden (r=0.63; p<0.01), higher Dermatology Life Quality Index (r=0.33; p<0.05), and lower mental quality of life (r=–0.30; p<0.05). Patients had a higher level of skin shame (F=74.03; eta2= 0.36; p<0.01) and less physical quality of life on the SF-36 (F=5.14; eta2= 0.04; p<0.05) than non-dermatological controls. General shame was not related to disease burden or quality of life. While self-rated skin shame appears to be related to quality of life in patients with psoriasis, no association was registered between expert-rated Psoriasis Area and Severity Index and quality of life.

Systemic mastocytosis (SM) is frequently associated with eosinophilia. To examine its prevalence and clinical impact in all WHO classification-based subcategories, we analyzed eosinophil counts in 2350 mastocytosis patients using the dataset of the European Competence Network on Mastocytosis. Ninety percent of patients had normal eosinophil counts, 6.8% mild eosinophilia (0.5–1.5 × 10 9 /l), and 3.1% hypereosinophilia (HE; >1.5 × 10 9 /l). Eosinophilia/HE were mainly present in patients with advanced SM (17%/19%), and only rarely recorded in patients with indolent and smoldering SM (5%/1%), and some patients with cutaneous mastocytosis. The eosinophil count correlated with organomegaly, dysmyelopoiesis, and the WHO classification, but not with mediator-related symptoms or allergy. Eosinophilia at diagnosis had a strong prognostic impact ( p < 0.0001) on overall survival (OS) and progression-free survival (PFS), with a 10-year OS of 19% for patients with HE, 70% for those with mild eosinophilia, and 88% for patients with normal eosinophil counts. In 89% of patients with follow-up data ( n = 1430, censored at start of cytoreductive therapy), eosinophils remained stable. In those with changing eosinophil counts (increase/decrease or mixed pattern), OS and PFS were inferior compared with patients with stable eosinophil counts. In conclusion, eosinophilia and HE are more prevalent in advanced SM and are predictors of a worse outcome.

ZusammenfassungIn der Vergangenheit haben Studien gezeigt, dass sich Interventionen bei chronisch Kranken und Krebspatient/-innen positiv auf die religiös-spirituelle (r/s) Befindlichkeit und die Krankheitsverarbeitung auswirken können. Es gibt jedoch keine standardisierten Vorgaben für r/s Interventionen. In einer Pilotstudie führten wir bei 10/24 dermatologischen Patient/-innen religiös-spirituelle Interventionen durch, während 7 Patient/-innen einen Fachvortrag mit anschließendem Telefoninterview erhielten. Die Kontrollgruppe (n=7) erhielt keine Intervention. Alle Patient/-innen wurden mittels des Multidimensionalen Inventars zum religiös/spirituellen Befinden (MI-RSB) sowie mit dem SF-36 zur Lebensqualität zweimalig befragt.Die r/s Interventionen fanden einmal pro Woche innerhalb von 2 Monaten insgesamt achtmal statt, dauerten 90 Minuten und wurden von einer christlichen Theologin vorgenommen, die Erfahrung in Exerzitien und spiritueller Begleitung hat. Elemente der Übungen waren Meditation, Rituale, Lieder, Erfahrungsaustausch, Geschichten und Impulse zu Themen, die für Sinnstiftung und die Tiefendimensionen des Lebens relevant sind. Diese Interventionen stützten sich auf Aspekte der ignatianischen Spiritualität und auf Grundfragen christlicher Spiritualität.Während jede Art von Zuwendung für die Patient/-innen eine Zunahme der Lebensqualität bedeutet, nimmt durch die r/s Interventionen die transzendente Hoffnung signifikant zu. Die Auswirkung unserer r/s Intervention könnte Anlass sein, derartige Interventionen weiterzuentwickeln und in größer angelegten Multicenter-Studien zu testen.

To prepare a dermatologic addendum to the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides (CHCC2012) to address vasculitides affecting the skin (D‐CHCC). The goal was to standardize the names and definitions for cutaneous vasculitis.

Objective: To prepare a dermatologic addendum to the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides (CHCC2012) to address vasculitides affecting the skin (D-CHCC). The goal was to standardize the names and definitions for cutaneous vasculitis. Methods: A nominal group technique with a facilitator was used to reach consensus on the D-CHCC nomenclature, using multiple face-to-face meetings, e-mail discussions, and teleconferences. Results: Standardized names, definitions, and descriptions were adopted for cutaneous components of systemic vasculitides (e.g., cutaneous IgA vasculitis as a component of systemic IgA vasculitis), skin-limited variants of systemic vasculitides (e.g., skin-limited IgA vasculitis, drug-induced skin-limited antineutrophil cytoplasmic antibody-associated vasculitis), and cutaneous single-organ vasculitides that have no systemic counterparts (e.g., nodular vasculitis). Cutaneous vasculitides that were not included in the CHCC2012 nomenclature were introduced. Conclusion: Standardized names and definitions are a prerequisite for developing validated classification and diagnostic criteria for cutaneous vasculitis. Accurate identification of specifically defined variants of systemic and skin-limited vasculitides requires knowledgeable integration of data from clinical, laboratory, and pathologic studies. This proposed nomenclature of vasculitides affecting the skin, the D-CHCC, provides a standard framework both for clinicians and for investigators.

Serum levels of the IFN-regulated cytokine CXCL13 have been found to correlate with SLEDAI and renal involvement in systemic lupus erythematosus. This study investigates whether CXCL13 can also be a marker of disease activity in patients with subacute cutaneous or chronic cutaneous lupus erythematosus (SCLE, CCLE). We analysed CXCL13 levels in 60 patients’ sera (18 SLE, 19 SCLE, 23 CCLE) at five time points within 1 year and correlated these levels with disease activity scores and laboratory markers. Clinical scores with no/mild, moderate or high/severe disease activity were categorized by SLEDAI in SLE, by CLASI in SCLE/CCLE. CXCL13 levels were significantly higher in SLE (median 122.5, IQR 88.0–239.0 pg/ml) than in CCLE patients (median 69.0, IQR 60.0–102.0 pg/ml) (p = 0.006). CXCL13 levels were elevated in 59% (41/70) of SLE patient visits with mild or no disease activity, but in 90% (9/10) with high disease activity. CXCL13 levels correlated with ECLAM, dsDNA-antibodies, and inversely with complement factors C3 and C4 in SLE, and with IgA and ESR in SCLE. In CCLE CXCL13 did not correlate with CLASI or laboratory markers. One SCLE and two CCLE patients with CXCL13 levels > 500 pg/ml had conversion to SLE or an underlying autoimmune disease. CXCL13 seems to be a useful marker of disease activity in SLE, but not in SCLE and CCLE. Conversion from normal to elevated CXCL13 may indicate a flare of SLE. Whether high CXCL13 levels in cutaneous LE indicate the development of SLE should be further investigated.

Background Systemic sclerosis (SSc) is a very heterogeneous multisystem connective tissue disease. The majority of affected patients develop initial clinical symptoms between the age of 30 to 50 years. It is not known whether an ageing population affects the clinical phenotype of SSc. Objectives To investigate the relationship of the age at disease onset and clinical characteristics in SSc patients using the registry of the German Network for Systemic Scleroderma. Methods Clinical data of the patient registry, currently including 4021 patients, were evaluated. Three age ranges at disease onset (<40 years, 40–60 years, and >60 years) were correlated with clinical characteristics. Results Among all SSc patients, 27% of patients developed first non-raynaud symptoms at the age <40 years, while 44% developed SSc between 40–60 years, and 29% were older than 60 years of age. In particular, SSc patients with disease onset >60 years developed significantly (p<0.001) more often the lcSSc subtype (71.1%), anti-centromere antibodies (45.8%), had a significantly lower modified Rodnan Skin Score (mRSS) (7.8±8.1), more often pulmonary hypertension (PH) (17.4%), a significantly lower mean DLCO level (69.7%±21.5) and less often digital ulcerations (20.2%). However, a significant difference for lung fibrosis, heart or kidney involvement could not be observed. The more progressive subsets (dcSSc and SSc overlap syndromes) are found significantly more often at younger ages (p<0.001). Conclusions In this registry, nearly one third of patients developed SSc at an age above 60 years. These are mostly of the limited cutanous subtype with frequent PH. These findings have an important influence on recommendations on diagnosis and theray of SSC. Disclosure of Interest None declared